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Improvement in echocardiographic and biomarkers after glucocorticoid therapy in infants with pulmonary hypertension

Presented By:

Brian Hernandez, BS; Rod Shinozaki, MD; Manish Aggarwal, MD; R. Mark Grady, MD; Andrea Drussa, CPNP; Hongjie Gu

Washington University School of Medicine

Brianh@wustl.edu

Overview:

Background/Hypothesis: Pulmonary hypertension (PH) in children is often associated with significant morbidity and mortality. Data in studies have shown the important role inflammation may play in the pathogenesis of PH. Limited evidence exists on anti-inflammatory treatment in PH. We assessed the effect of treating PH in infants with glucocorticoids using echocardiographic changes and diagnostic biomarkers as measures of efficacy.

Materials & Methods: A retrospective chart review was performed on hospitalized infants <1 year of age at St. Louis Children’s Hospital with 12 (70%) WHO Group 3 PH and 5 (30%) WHO Group 1 PH who received a 5-7 day course of systemic glucocorticoid treatment from January 2017 to December 2021. Quantitative echocardiographic indices for PH, N-terminal pro-hormone brain natriuretic peptide (NT-proBNP) and/or BNP levels were collected pre and post-glucocorticoid treatment.

Results: Seventeen infants met inclusion criteria:<1 year old, no significant intracardiac shunts, and evidence of elevated right ventricular pressures. There were eight (47%) males, a mean (+/-SD) gestational age of 32.1 (+/-5.8) weeks, and three (17%) concomitantly treated with sildenafil. Results showed, BNP significantly decreased (p<0.0001), pulmonary artery acceleration time (PAAT) increased (p=0.0015), and PAAT to right ventricular ejection time ratio (PAAT/RVET) increased (p=0.0006). No significant difference was found with eccentricity ratio, tricuspid annular plane systolic excursion, or tricuspid regurgitation (TR) jet. Fifteen of seventeen infants had insufficient TR jet envelopes for analysis.

Conclusions: In our retrospective study, systemic glucocorticoid therapy was associated with significant improvement in BNP levels, PAAT, and PAAT/RVET ratio in infants with WHO Group 1 and 3 PH. These results provide impetus for further prospective studies in how glucocorticoids may provide benefit to this population of affected children.